Management of Hemophilic Cysts and Pseudotumors of the Hand in Bleeding Disorders: A Case Series.

PURPOSE: Hemophilic cysts and pseudotumors (HCPTs) of the hand are rare and are secondary to bleeding disorders such as hemophilia A and B. This is a report of our experience in the management of this rare condition.

PATIENTS AND

METHODS

Seven male patients with hemophilia A presenting with progressive swelling of the hand were treated between 2004 and 2013 at a tertiary referral hospital. All patients had clotting factor replacement based on our previously reported protocol. The age of the patients ranged from 3 to 49 years (median age, 19 years).

RESULTS

Four patients had soft tissue hemophilic cysts and 3 had bony hemophilic pseudotumors. Two patients had traumatic pseudoaneurysm of the ulnar artery in addition to the cysts. The soft tissue cysts required surgical excision in 3 patients under factor cover as per the protocol. The bony lesions were initially managed nonsurgically by factor replacement, but 2 patients failed to respond and required amputation of the fingers. The ulnar artery aneurysm was excised and artery ligated in 1 patient and the artery was vein grafted owing to poor hand perfusion in 1.

CONCLUSIONS

Based on our observations in the management of HCPTs of the hand and the existing literature, we conclude that the soft tissue cysts require surgical excision along with factor replacement and distal bony lesions smaller than 3 cm respond to factor replacement. Larger bony lesions require surgical treatment. Treatment of hemophilic cysts and pseudotumors should be undertaken only in centers with a major hematology backup.

TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic V.